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78 Cards in this Set
- Front
- Back
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Adrenal incidentaloma
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0.4-4.4%
Benign adenomas (60%) Secondary mets (19%) Primary adrenocortical malignancies (8%) Myelipomas (9%) Pheos (10%) |
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Work up of adrenal incidentaloma
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check 24 hour urinary cathecholamine, serum K, urinary hydroxycorticosteroids
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Primary tumors that metastasize to adrenal
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Breast CA (#1)
Melanoma Renal CA Lung CA |
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Workup of hypertensive pt with adrenal mass
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1) Overnight 1 mg dexamethasone suppression test or 24 hour urinary cortisol
2) 24 hour urinary catecholamine 3) PAC/PRA (check for aldosteronoma) |
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Pheochromocytomas in pregnancy
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normal urine protein cf pre-eclampsia
most effective screening study --> MRI |
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Management of pheochromocytoma in pregnany women
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alpha blockage and adrenalectomy no matter what trimester except for third (can wait after C section - no vaginal delivery no matter what!)
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MEN 1
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parathyroid hyperplasia
pancreatic/duodenal neuroendocrine tumors (30-80%) anterior pituitary adenomas |
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How do you rule out duodenal neuroendocrine tumors?
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open duodenotomy
intraop endoscopic intubation with transillumination |
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Most common functional neuroendocrine tumor of pancreas in MEN 1
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Gastrinoma (high recurrence)
2nd MC --> insulinoma (lower recurrence) |
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Hurthle Cell (oncocytic tumors)
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Rare thyroid CA originating w/n follicular cell of thyroid
<5% of all differentiated carcinomas of thyroid glands occurs in older population (60) high post op recurrence during 1st 2 decades and after age 60 |
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Prognosis of lymph node metastasis in well differentiated thyroid CA
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poorer prognosis in Hurthle cell but not in follicular or papillary
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Sex differences in thyroid pap/foll CA
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women twice as likely to develop
Men twice as likely to die |
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Strongest risk factor for developing thyroid CA
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history of goiter (>5yrs)
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Hypocalcemia Si/Sx
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perioral numbness
tingling of fingers muscle cramps anxiety Chvostek's sign Trousseau's sign |
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Treatment of hypocalcemia
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oral calcium for mild sx
add calcitriol for moderate IV Ca for severe |
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Adrenocortical CA
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usually large when found (9-12cm)
40-50 y.o 1/million heterogeneous mass central necrosis irregular shape/margins 2/3 are biochemically active |
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Most commonly produced hormones in functioning adrenocortical CA
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1.) Cortisol
2.) Sex 3.) Aldosterone Mixed in 35% |
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Management of chronic steroids undergoing major emergency surgery
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stress dose of 150mg/day in divided doses for 2-3 days
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The four Ds of glucagonoma
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Diabets
Dermatitis DVT Depression |
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Necrolytic migratory erythema
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Pathognomonic for glucagonoma
70% @ time of dx Erythematous plaques on face, abd, groin, LE with central clearing and blistering/encrusted borders |
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work up and Treatment of glucagonomas
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Check chromogranin A & glucagon
CT abd/pelvis Surgical resection/debulking |
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Laparoscopic adrenalectomy
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Lateral decubitus is superior to supine
Used for pheochromocytomas Not used to remove adrenocortical cancer |
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Management of substernal goiter
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Resection if symptomatic (SVC syndrome)
Thyroidectomy via Kocher incision |
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60M w/ 4cm left papillary thyroid CA. Management?
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FNA (98-100%)
total thyroidectomy + ablative I 131 & thyroxine (latter reduces recurrence for tumors <1cm) |
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Medullary thyroid CA
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Derived from parafollicular C cells
5-10% of all thyroid Ca total thyroidectomy with central lymph node dissection |
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MEN2A
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MTC
Pheo (50%) Hyperparathyroidism (20%) |
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MEN 2B
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MTC
Marfanoid habitus mucosal neuromas |
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MEN1
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MENIN gene mutation (cf ret-protoconcogene)
parathyroid hyperplasia neuroendocrine tumors of pancreas/duodenum anterior pituitary adenoma |
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Unilateral aldosterone producing adrenal mass with normal CT findings. Next step?
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adrenal vein sampling
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Somatostinoma presentation
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1.) Mild DM
2.) Gallstones 3.) Diarrhea +/- steatorrhea |
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Glucagonoma presentation
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Necrolytic migratory erythema
Cheilitis DM Anemia Weight loss Venous thrombosis Neuropsychiatric symptoms |
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Insulinomas....benign or malignant usually?
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benign (10% mal)
Vipomas 50% mal Gastrinomas 60% mal |
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VIPoma presentation
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VIP (28 AA polypeptide) binds to intestinal epithelial cells activating cellular adenylate cyclase and cAMP leading to
1.) secretory Watery Diarrhea 2.) Hypokalemia 3.) Hypochlorhydria 4.) Alkalosis |
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Primary Hyperaldosteronism
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high aldo low renin
Aldosteronoma 60% Idiopathic hyperaldosteronism 30% |
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Aldosteronoma
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60 of primary hyperaldosteronism
younger, women More severe HTN & HypoK No effect from salt loading or postural changes AVS --> unilateral hypersecretion of aldosterone |
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Idiopathic hyperaldosteronism
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30% of primary hyperaldosteronism
Older, men Salt loading decreases aldo Postural testing increases aldo AVS bilateral hypersecretion Tx: spironolactone |
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When do you have to biopsy an adrenal incidentaloma?
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if there is prior history of CA
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Management of <3cm adrenal incidentaloma
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f/u in 6 months
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Approach for andrenal CA resection
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anterior
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What enzymes are in all zones of adrenal cortex?
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21 and 11 beta hydroxylase
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Actions of aldosterone
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absorb sodium at the expensive of K, H, NH3
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What stimulates aldosterone secretion?
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Ang II, hyperkalemia, ACTh (less)
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Congenital adrenal hyperplasia...most common?
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21-hydroxylase deficiency (90%)
Salt wasting Hypotension Precocious puberty in males and virilization in females increased 17-OH progesterone |
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Which congenital adrenal hyperplasia causes hypertension?
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11 hydroxylase deficiency
increased 11-deoxycortisone |
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Causes of Conn's syndrome
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Primary (low renin) adenoma (80-90%), hyperplasia 10-20%
Secondary disease - high renin, more common |
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Workup for hyperaldosteronism
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Urine Aldosterone after salt load test
increased urine K, serum NA Low serum K metabolic alkalosis |
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What is diagnostic for primary aldosteronism
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Aldosterone:renin >400
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Localizing studies for hyperaldo
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MRi
NP-59 scinotography adrenal venous sampling |
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tumor vs hyperplasia in conn's syndrome
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Captopril test
upright posture 18-OH corticosterone |
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Treatment for hyperaldosteronism
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Adenoma --> resect
Hyperplasia --> medical tx first with spironolactone, CCB, potassium (if refractory --> b/c resxn and fludrocortisone) |
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#1 cause of Addison's disease
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w/d of exogenous steroids
autoimmune dx with ACTH stim test |
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Cushing's syndrome
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MC iatrogenic
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How to dx Cushing's
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1.) 24 hr urine cortisol
2.) low dose dexamethasone test 3.) Measure ACTH 4.) High dose dexa 5.) CRH test (pit.adenomas will increase ACTH, ectopic won't) |
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#1 noniatrogenic cause of Cushing syndrome
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Pituitary adenoma 70-80%
mostly microadenomas petrosal sampling to determine side cortisol supp with low/high dex Tx: transsphenoidal or XRT for unresectable |
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#2 noniatrogenic cause of Cushing's syndrome
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Ectopic ACTH
MC small cell lung CA Not suppressed with dex |
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#3 noniatrogenic cause of Cushing's syndrome
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adrenal adenoma
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Treatment of adrenocortical CA
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radical adrenalectomy
debulking needed |
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When do you operate on adrenal incidentaloma?
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<5cm
Functional nonhomogenous margins attenuation growing |
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When is adrenal venous sampling indicated?
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primary hyperaldosteronism has been confirmed and thin-cut adrenal CT reveals either no abnormalities or bilateral abnormalities
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Cushing's syndrome features
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obesity
hirsutism amenorrhea easy bruising extreme muscle weakness |
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Preoperative management of adrenalectomy for cushing's syndrome
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preoperative stress dose of steroids
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treatment of adrenocortical CA
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radical adrenalectomy
en-bloc |
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Chemo for adrenocortical carcinoma?
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Mitotane
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Classic Triad of Pheo
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Headache
Palpitations Diaphoresis |
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Work up for pheo
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free plasma metanephrine
24h urine cathecholamines/metabolites |
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What is adrenal medulla derived from?
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Ectoderm neural crest cells
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Catecholamine production
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tyrosine->dopa->dopamine->norepinephrine->epinephrine
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Rate limiting step in catecholamine production?
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tyrosine hydroxylase
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Perioperative management of pheo
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2 weeks of preop phenoxybenzamine
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What enzyme is found only in adrenal medulla?
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PNMT
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Where is extraadrenal tissue usually found?
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Organ of Zuckerkandl
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What is pheochromocytoma associated with?
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MENIIa, IIB, von recklinghausen's dz, tuberous sclerosis, sturge-weber
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10% rule of pheo
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malignant (esp when extraadrenal)
bilateral in children familial extraadrenal |
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Dx of pheo
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urine metanephrine and VMA (most sensitive)
clonidine suppression test No venography |
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which drug inhibits thyrosine hydroxylase
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metyrosine
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what do you ligate first in adrenalectomy for pheo?
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adrenal veins
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Other sides of pheo
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vertebral bodies, opp adrenal, bladder, aortic bifurcation
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What is common to both MEN I and IIA
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Parathyroid hyperplasia
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